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Rheuma > Rheuma > Flashcards

Rheuma Flashcards

1
Q

most common rheumatic disease in children

A

Juvenile Idiopathic Arthritis (JIA)

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2
Q

criteria for JRA

A

age onset <16
arthritis (swelling or effusion or presence of 2 or more of the ff signs: limitation of range of motion, tenderness or pain on motion, increased heat) in >/= 1 joints
duration of >6weeks
polyarthritis >/=5 inflamed joints or
oligoarthritis =4 joints
exclusion of other forms of juvenile arthritis

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3
Q

systemic onset JIA

A 
arthritis >/=1 joint with or preceded by fever of atleast 2 weeks documented to be quotidian for atleast 3 days
and accompanied by >/=1 of the ff:
*evanescent (nonfixed) erythematous rash
*generalized LN enlargement
*Hepatomegaly or splenomegaly or both
*serositis
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4
Q

Oligoarticular JIA

A

arthritis affecting 1-4 joints during 1st 6 months of disease

  • persistent- <4joints
  • extended- >5 joints
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5
Q

Psoriatic arthritis

A

Arthritis and Psoriasis or arthritis at least 2 of the ff:

  • dactylitis
  • nail pitting and oncholysis
  • psoriasis in a 1st degree relative
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6
Q

what is the risk for being ANA positive in patients with JIA?

A

increased risk for anterior uveitis

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7
Q

treatment for JIA

A

NSAIDs
Methotrexate
TNF-a antagonist

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8
Q

symptoms characteristic of inflammatory back pain

A

pain at night with morning stiffness
no improvement with rest
improvement with exercise
insidious onset

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9
Q

strongest risk factor for SLE

A

female

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10
Q

histopathologic feature most suggestive of SLE

A

discoid rash

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11
Q

hallmark of SLE

A

generation of autoantibodies directed against self-atigens

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12
Q

most feared complication of neonatal lupus

A

congenital heart block

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13
Q

heliotrope rash

A

blue-violet discoloration of eyelids

seen in Juvenile dermatomyositis

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14
Q

What are Gottron papules?

A

bright pink or pale, shiny thickened or atrophic plaques over proximal interphalangeal joints and DIP
Juvenile dermatomyositis

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15
Q

affects the proximal muscles with symmetric muscle weakness

A

Juvenile dermatomyositis

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16
Q

how do we diagnose Juvenile dermatomyositis?

A 
characteristic rash and at least 3 of the ff:
muscle weakness
muscle enzyme elevation
EMG changes(myopathy, denervation)
muscle biopsy (necrosis, inflammation)
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17
Q

what is en coupe de sabre

A

morphea confined to frontal scalp, forehead and midface in a linear band may have seizure, hemifacial atrophy, ipsilateral uveitis, learning and behavioral changes (Scleroderma)

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18
Q

most common visceral manifestation of Scleroderma

A

Pulmonary disease

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19
Q

also known as mucocutaneous lymph node syndrome or infantile polyarteritis nodosa

A

Kawasaki disease

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20
Q

Kawasaki disease affects what arteries?

A

medium-sized arteries

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21
Q

most important manifestation of Kawasaki disease

A

cardiac involvement

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22
Q

wHat are the 3clinical phases of Kawasaki Disease

A

Acute febrile phase which lasts 1-2 weeks
Subacute phase: desquamation, thrombocytosis, developmentof coronary aneurysms lasts about 2 weeks
Convalescent phase:all clinical signs have disappeared and continues until ESR returns to normal

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23
Q

In Kawasaki disease, when do we expect the platelet count to increase?

A

second to third week of illness

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24
Q

In Kawasaki Disease, when echocardiography be done?

A

at diagnosis and at 2-3 weeks of illness

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25
Q

Treatment for Kawasaki Disease

A

IVIg 2g/kg
High dose Aspirin (80-100mkday q6) within 10 days onset
decrease dose of Aspirin to 3-5mg/kg/day as single dose after being afebrile for 48 hours and continued until 6-8 weeks after illness onset

26
Q

In patients who had Kawasaki disease and was given IVIG, when can we give live vaccines?

A

After 11 months

27
Q

most common vasculitis of childhood

A

HSP

28
Q

hallmark of HSP

A

rash: palpable purpura starting as pink macules or wheals developing into petechiae, raised purpura or larger ecchymoses

29
Q

Classification criteria for HSP

A

ACR criteria: at least 2 of the ff:

  • palpable purpura
  • age onset <20
  • bowel angina
  • biopsy demonstrating intramural granulocytes in small arterioles and/or venules

European League against Rheumatism

  • palpable purpura
  • diffuse abdominal pain
  • arthritis or athralgia
  • biopsy of affected tissue demonstrating predominant IgA deposition
30
Q

treatment for HSP

A

steroids

31
Q

“pulseless disease”

A

Takayasu arteritis

*subclavian, renal and carotid arteries mostly involved

32
Q

criteria for Pediatric onset-takayasu arteritis

A

angiographic abnormalities of the aortaor its main branches and at least one of the ff:

  • decreased peripheral artery pulse and/or claudication
  • BP difference between arms or legs >10mmHG
  • bruits over aorta or its major branches
  • hypertension
33
Q

mainstay of therapy for Takayasu

A

Glucocorticoids

34
Q

necrotizing vasculitis affecting the small and medium sized arteries

A

Polyarteritis nodosa

*conventional arteriography is the gold standard

35
Q

found to be associated with neonatal lupus

A

Anti-Ro/SSA
Anti-La/SSB
Anti-RNP

36
Q

cut-off value for ANA to diagnose SLE

A

> /= 1:80

37
Q

What to do if mother is positive for anti-Ro and anti-la?

A 
2% offspring will develop neonatal lupus
2nd pregnancy 15-20%
*regular fetal echo at 16 weeks of gestation until delivery
*If (+) fetal incomplete block/hydrophic changes: 
fluorinated steroids
IVIg
plasmapharesis
hydroxychloroquine
Terbutaline + steroids given to mother
38
Q

prognosis of neonatal LE

A

skin, liver and hematologic complications generally resolve with minimal sequalae
All children with CAVB require pacemaker insertion by age of 15
heart block is permanent

39
Q

Criteria for diagnosis of Behcet’s Disease

A

recurrent oral ulcers plus 2 of the ff:

  • recurrent genital ulcers
  • eye lesions
  • skin lesions
  • pathergy - papular or pustular response after injection of NSS
40
Q

treatment for Behcet’s Disease

A

Azathioprine: eye disease
Steroids, sucralfate: oral and genital ulcers
Colchicine: erythema nodosum/arthritis (males), genital ulcers (females)
Steroids, azathioprine, cyclophosphamide, interferon alpha,TNF blockers: CNS disease

41
Q

Diagnosis of Immunoglobulin vasculitis (IgA)

A

Petechiae or purpura with lower limb predominance

Plus >/= 1/4 of the ff:
Diffuse abdominal colicky pain with acute onset (including intussusception and GI bleeding)
Skin biopsy showing leukocytoclastic vasculitis with IgA deposits
Arthritis or athralgia of acute onset
Renal involvement (proteinuria >0.3g in 24 hours, hematuria or RBC casts, impaired renal function

42
Q

common presenting complaint in JDM

A

Fatigue

43
Q

Most common inflammatory myositis in children distinguished by proximal weakness and characteristic rash

A

JDM

44
Q

Diagnostic criteria of JDM

A 
Classic rash plus 3 of the ff:
Weakness
Muscle enzyme elevation
Electromyographic changes
Muscle biopsy
45
Q

Blue violet discoloration of eyelids

A

Heliotrope rash seen in

JDM

46
Q

most common visceral manifestation of systemic sclerosis

A

Pulmonary disease

47
Q

most frequent initial symptom of in pediatric systemic sclerosis

A

Raynaud phenomenon

48
Q

vasospastic disorder resulting in cool, painless, bluish discoloration in the hands and sometimes feet despite normal tissue perfusion

A

Acrocyanosis

49
Q

most common cause of death in Scleroderma

A

heart failure

50
Q

confined to frontal scalp, forehead and midface in linear band

A

en coup de sabre

51
Q

most common rheumatic disease in children

A

JIA

52
Q

most common subtype of JIA

A

Oligoarthritis

53
Q

most common affected joints in Enthesitis-Related Arthritis

A

Knees, ankles and hips

54
Q

most common presenting complaints of children with SLE

A

fever, fatigue, hematologic abnormalities, arthralgia and arthritis

55
Q

most common cause of death in patients with SLE

A

infection, complications of GN and neuropsychiatric disease

56
Q

most common subtype of Juvenile localized scleroderma

A

Linear scleroderma

57
Q

most common manifestations of Sjogren syndrome in children

A

recurrent parotid gland enlargement and parotitis

58
Q

most common manifestations of Sjogren syndrome in adults

A

Sicca syndrome (dry mouth, painful mucosa, sensitivity to spicy foods, halitosis, widespread dental caries

59
Q

most common vessels affected in Kawasaki disease

A

coronary arteries

60
Q

aortic branches affected in Takayasu arteritis

A

subclavian
renal
carotid arteries

61
Q

most common presenting problem of children referred to pediatric rheumatology clinics

A

musculoskeletal pain

62
Q

most common cause of recurrent musculoskeletal pain in children

A

Benign nocturnal pains of chidlhood (growing pains)

Rheuma (1 decks)

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